Cystic fibrosis (CF), also known as mucoviscidosis, is a lethal common autosomal-recessive disorder. The disease is caused by mutations in the gene encoding the CF transmembrane conductance regulator (CFTR), an anion channel that helps maintain fluid and electrolyte-homeostasis in multiple organs, including the lungs and pancreas. The treatment options include: airway-clearance techniques and medications to clear mucus from the lungs; proactive treatment of infections; pancreatic enzyme replacement therapy (PERT); optimal nutrition; and an active lifestyle. Ivacaftor (Kalydeco) was the first drug approved by the US Food and Drug Administration (FDA) in 2012, however it remains very expensive. Other medications used to treat patients with cystic fibrosis may include the following: multivitamins (including fat-soluble vitamins), nebulized, inhaled, oral, or intravenous antibiotics, bronchodilators, anti-inflammatory agents, agents to treat associated conditions or complications (insulin, bisphosphonates), and agents devised to potentially reverse the abnormalities in chloride transport (ivacaftor (Kalydeco), lumacaftor/ivacaftor). A number of promising CF products with different modes of action are in the developmental pipeline. The candidate drugs that remain on the current development pipeline include Ataluren (Translarna), CFTR gene therapy, Lynovex, Alpha‑1 antitrypsin, Sildenafil (Revatio), Levofloxacin (Aeroquin), Arikace (inhaled amikacin), AeroVanc (inhaled vancomycin), Liprotamase, OligoG, etc.